儿童重症抗NMDAR脑炎的临床特征、治疗及预后分析  

Clinical characteristics,treatment and prognosis of children with severe anti-N-methyl-D-aspartate receptor autoimmune encephalitis

作　　者：韩慰[1] 蒋莉[1] 洪思琦[1] 符跃强[2] 卢思为[2] Han Wei;Jiang Li;Hong Si-Qi;Fu Yue-Qiang;Lu Si-Wei(Department of Neurology,Children’s Hospital of Chongqing Medical University/National Clinical Research Center for Child Health and Disorders/China International Science and Technology Cooperation Base of Child Development and Critical Disorders/Chongqing Key Laboratory of Pediatrics,Chongqing 400014,China;Department of Pediatric Intensive Care Unit,Children’s Hospital of Chongqing Medical University,Chongqing 400014,China)

机构地区：[1]重庆医科大学附属儿童医院神经内科/国家儿童健康与疾病临床医学研究中心/儿童发育疾病研究教育部重点实验室/儿科学重庆市重点实验室,重庆400014 [2]重庆医科大学附属儿童医院重症监护室,重庆400014

出　　处：《解放军医学杂志》2022年第11期1125-1132,共8页Medical Journal of Chinese People's Liberation Army

基　　金：重庆市教委科学技术研究项目(KJQN202100423);儿童发育疾病研究教育部重点实验室基础研究青年项目(YBRP-202110)。

摘　　要：目的总结分析儿童重症抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的临床特征、治疗及预后情况。方法收集2014年1月-2021年9月重庆医科大学附属儿童医院神经内科及儿童重症监护病房(PICU)收治的20例重症抗NMDAR脑炎患儿的临床资料。分析所有患儿的临床特征、治疗及预后情况;采用改良Rankin量表(mRS)评估患儿的短期预后,将其分为预后良好组(mRS<2分)与预后不良组(mRS≥2分或死亡),并分析其预后影响因素。结果纳入的20例重症抗NMDAR脑炎患儿中,女11例,男9例,发病年龄(9.2±3.7)岁。首发神经系统症状以惊厥发作、精神行为异常为主,主要并发症为意识障碍(85.0%),癫痫持续状态(60.0%),中枢性低通气(40.0%),急性颅内压增高(35.0%),多脏器功能衰竭(20.0%),休克(20.0%),横纹肌溶解(15.0%),其中短期预后良好的患儿无多脏器功能衰竭、休克和横纹肌溶解发生。首次脑电图检查异常19例(95.0%),主要为慢波活动,短期预后良好的患儿均无痫样波及δ刷。所有患儿均接受一线治疗,包括甲泼尼龙、静脉用丙种球蛋白(IVIG)或血浆置换(TPE),2例患儿接受利妥昔单抗二线免疫治疗,免疫治疗前后GCS评分有所改善。出院后进行3个月的短期随访,发现4例患儿死亡,16例存活患儿遗留以认知功能减退(12例,75.0%)、语言障碍(10例,62.5%)及运动障碍(8例,50.0%)为主的神经系统功能障碍,mRS评分为(2.81±1.55)分。近期预后良好组入院到完善抗体检测的时间明显短于近期预后不良组(P=0.032)。结论儿童重症抗NMDAR脑炎除尽早完善抗体检测及脑电图检查、尽快启动免疫治疗外,还应重视呼吸、循环及癫痫持续状态的管理;大部分患儿免疫治疗有效,但近期预后欠佳。Objective To summarize and analyze the clinical features,treatment and prognosis of children with severe anti-N-methyl-D-aspartate receptor(anti-NMDAR)encephalitis.Methods A total of 20 cases clinical data of children patients with severe anti-NMDAR encephalitis were collected from the Department of Internal Neurology and Pediatric ICU of Children's Hospital affiliated to Chongqing Medical University from January 2014 to September 2021.The clinical features,treatment and prognosis were analyzed.Modified Rankin scale(mRS)was used to evaluate the short-term prognosis of the patients,and divided them into favourable prognosis group(mRS<2)and unfavourable prognosis group(mRS≥2,or death),and analyze the influencing factor to prognosis.Results A total of 20 children with severe anti-NMDAR encephalitis were included,among them 11 girls and 9 boys with on-set age of(9.2±3.7)years.convulsion attack and mental behaviour disorder were the first neurological symptoms,and the main complications included consciousness disturbance(85.0%),status epilepticus(60.0%),central hypoventilation(40.0%),acute intracranial hypertension(35.0%),multiple organ dysfunction(20.0%),shock(20.0%)and rhabdomyolysis(15.0%),while no multiple organ dysfunction,shock and rhabdomyolysis occurred in favourable prognosis group.Moreover,slow wave activity was critical feature of first electroencephalography(EEG)abnormalities(95.0%),and none of the patients in favourable prognosis group had epileptiform waves and δ-brushes.The first-line immune treatment was received by all the children patients including the combination therapies of methyllprednisolone,intravenous immunoglobulin(IVIG)or plasma exchange(TPE),among them 2 cases received the second-line immunotherapy with rituximab.Glasgow coma score(GCS)scores improved after immunotherapy compared with pre-treatment.Short-term follow-up was performed for 3 months.Finally,4 of the children patients died and 16 survived with neurological deficits,mainly including cognitive impairment(12 cases,75.0%),langu

关 键 词：N-甲基-D-天冬氨酸受体(NMDAR) 儿童 重症脑炎 抗NMDAR脑炎 

分 类 号：R593.2[医药卫生—内科学] R720.5[医药卫生—临床医学]